Hypertrophic cardiomyopathy in children: Management and prognosis
- John Jefferies, MD, MPH, FACC, FAHA
John Jefferies, MD, MPH, FACC, FAHA
- Professor, Pediatric Cardiology and Adult Cardiovascular Diseases
- The Heart Institute
- Professor, Division of Human Genetics
- Cincinnati Children’s Hospital Medical Center
- Thomas D Ryan, MD, PhD
Thomas D Ryan, MD, PhD
- Assistant Professor of Pediatrics
- The Heart Institute, Cincinnati Children's Hospital Medical Center
- Martin S Maron, MD
Martin S Maron, MD
- Assistant Professor of Medicine
- Tufts University School of Medicine
- Section Editor
- John K Triedman, MD
John K Triedman, MD
- Section Editor — Pediatric Cardiology
- Professor of Pediatrics
- Harvard Medical School
- Deputy Editors
- Brian C Downey, MD, FACC
Brian C Downey, MD, FACC
- Deputy Editor — Cardiology
- Assistant Professor of Medicine
- Tufts University School of Medicine
- Carrie Armsby, MD, MPH
Carrie Armsby, MD, MPH
- Senior Deputy Editor — UpToDate
- Deputy Editor — Pediatrics
- University of Massachusetts School of Medicine
Hypertrophic cardiomyopathy (HCM) is one of the most common forms of inherited cardiomyopathy in both adults and children, and it is characterized by hypertrophy of the left ventricle (LV) which sometimes involves the right ventricle. The disease course is highly variable but it is well recognized that there is an increased risk of morbidity and sudden cardiac death (SCD). (See "Sudden cardiac arrest and death in children".)
In broad terms, the symptoms related to HCM can be categorized as those related to heart failure, chest pain, or arrhythmias. Patients with HCM have an increased incidence of both supraventricular and ventricular arrhythmias and are at an increased risk for SCD. Overall, age at death has a bimodal distribution with the highest frequencies in infancy and adolescence, and the poorest survival in patients with inborn errors of metabolism and malformation syndromes diagnosed before one year of age . HCM is the most common cause of SCD in young, athletic, seemingly healthy individuals, accounting for more than one-third of SCD cases . (See "Hypertrophic cardiomyopathy: Prevalence, pathophysiology, and management of concurrent atrial arrhythmias" and "Hypertrophic cardiomyopathy: Assessment and management of ventricular arrhythmias and sudden cardiac death risk".)
Importantly, no medical treatments have been shown to alter disease progression. Management strategies are focused on symptom improvement, with utilization of potentially life-saving therapy in the form of implantable cardioverter defibrillators (ICDs) in patients deemed to be at high risk of SCD.
This topic will provide an overview of the management and prognosis of HCM in children. The clinical manifestations and diagnosis of HCM in children are discussed separately. (See "Hypertrophic cardiomyopathy in children: Clinical manifestations and diagnosis".)
The clinical manifestations, diagnosis, management, and natural history of HCM in adults are discussed separately:
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- Treatment goals
- General principles of medical therapy
- Pharmacologic therapy
- - Patient selection
- - First-line agents
- - Second-line agents
- - Combination therapy
- Persistent heart failure symptoms
- Arrhythmia treatment and prevention of sudden cardiac death
- - ICD therapy
- - Therapies other than ICD implantation
- LONG-TERM HEALTH MAINTENANCE
- Monitoring of growth parameters
- Monitoring for cardiac symptoms
- Antibiotic prophylaxis
- Exercise and sports participation
- Planning of non-cardiac surgery
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS